A 22-months- old girl presented to the outpatient department with poor weight gain and recurrent respiratory tract infection since early infancy. There was mild cyanosis at rest and oxygen saturation by pulse oximetry was 85%. Cardiac examination revealed normal S1, loud S2 with faint systolic murmur at left sternal border. Chest X ray showed mild cardiomegaly (CTR 60%) with increased pulmonary blood flow. Echocardiogram demonstrated situs solitus, levocardia, large ASD and a large VSD. There was severe PAH as suggested by low velocity gradient across large perimembranous VSD. The left ventricle was dilated (z score +2.2).
Although the shunt across VSD was bidirectional, it was predominantly left-to- right. Moreover, the reversal was not significant and the child was not old enough to explain visible cyanosis at rest (GIF 1 & Video 1). A careful assessment indicated patency of LSVC. Though there was high suspicion, the exact site of drainage of LSVC could not be confirmed. Subsequently performed injection of agitated saline from the left arm demonstrated first appearance of microbubbles in the left atrium
(GIF 2, 3 & Video 2, 3https://drive.google.com/drive/folders/0B1lviGfDpBvjSFEySFhSUE5kVU0).
This was followed by opacification of left ventricle and right ventricle. Saline contrast echocardiography thus substantiated the diagnosis of anomalous drainage of LSVC to LA and obviated the need additional imaging.
The index case supports well recognized role of saline contrast echocardiography in the diagnosis of anomaly of systemic venous connection. 1-4 This brief report also emphasizes the importance of comprehensive assessment. The timely recognition of anomalies of systemic venous connection is extremely important. Without the diagnosis of LSVC to LA this child ran the risk of having a diagnosis of inoperable hemodynamics with all undesirable consequences.
References
Gupta SK, Shetkar SS, Ramakrishnan S, Kothari SS. Saline contrast echocardiography in the era of multimodality imaging – Importance of “bubbling it right”. Echocardiography 2015;32:1707?19.
Shetkar SS, Gupta SK, Gulati GS, Juneja R. Right superior vena cava to left atrium: Importance of “bubbling it right”. Echocardiography 2014;31:E161?2.
Gupta SK, Gupta A. Saline contrast echocardiography for the detection of anomalous origin of pulmonary artery from aorta. Echocardiography 2017;34:145?6.
Gupta SK, Juneja R, Anderson RH, Gulati GS, Devagorou V. Clarifying the anatomy and physiology of totally anomalous systemic venous connection. Ann Pediatr Card 2017;10:269-77.